🧒🟡🧬 Primary Sclerosing Cholangitis (PSC) in Children: A Parent-Friendly Guide

✅ Primary sclerosing cholangitis (PSC) is a condition where the body’s immune system causes inflammation and scarring in the bile ducts (the “drainage pipes” that carry bile from the liver to the intestine).
PSC often overlaps with inflammatory bowel disease (especially ulcerative colitis) and needs long-term monitoring.

1) 🧾 Quick “At-a-glance” box (top of page)

✅ Condition name: Primary Sclerosing Cholangitis
Common names: PSC, bile duct inflammation/scarring

Plain-language summary (2–3 lines):
PSC causes bile ducts to become inflamed and narrowed. This can reduce bile flow and irritate the liver over time. Many children feel well early, but regular follow-up is important because PSC can flare and can lead to complications in some cases.

Who it affects (typical ages):
Can occur in school-age children and teens. Often diagnosed during workup for abnormal liver tests or in children with IBD.

✅ What parents should do today:

• Keep all hepatology and IBD follow-ups (if applicable)
• Ask which monitoring plan your child needs (blood tests, imaging, colonoscopy schedule)
• Watch for jaundice, itching, fevers, belly pain
• Ensure vaccines are up to date (especially hepatitis)

⚠️ Red flags that need urgent/ER care:

• High fever with chills and worsening right-upper belly pain (possible bile duct infection)
• Yellow eyes/skin worsening quickly
• Severe sleepiness/confusion
• Vomiting blood or black stools

🟡 When to see the family doctor/clinic:

• New itching, fatigue, or belly discomfort
• Recurrent fevers
• Abnormal liver tests
• New jaundice

2) 🧠 What it is (plain language)

Bile ducts are tubes that carry bile:

• from the liver
• through the bile ducts
• into the intestine to help digest fats

In PSC:

• bile ducts become inflamed
• scarring forms
• ducts can narrow (like a “pinched straw”)
• bile backs up and can irritate the liver

What part of the body is involved? (small diagram required)

Common myths vs facts

• Myth: “PSC is caused by diet.”
  Fact: PSC is an immune-related disease; diet does not cause it.
• Myth: “If my child feels fine, PSC is not serious.”
  Fact: PSC can be silent early; monitoring prevents missed complications.
• Myth: “PSC always leads to transplant.”
  Fact: Some children remain stable for many years; outcomes vary.

3) 🧩 Why it happens (causes & triggers)

Cause

• Immune-mediated inflammation of bile ducts (exact cause unknown)

Strong associations

• inflammatory bowel disease (IBD), especially ulcerative colitis

Triggers that can worsen symptoms (flares)

• infections
• inflammation activity (often overlaps with gut inflammation)
• bile duct blockage episodes

Risk factors

• personal or family history of autoimmune disease
• IBD diagnosis

4) 👀 What parents might notice (symptoms)

Many children have few symptoms early.

Possible symptoms

• fatigue
• itching
• intermittent belly discomfort
• jaundice (yellow eyes/skin)

Symptoms of bile duct infection (urgent)

• fever + chills
• worsening right-upper belly pain
• jaundice worsening quickly
• vomiting or appearing very unwell

What’s normal vs what’s not

✅ Stable PSC:

• child feels well
• labs stable with follow-up

⚠️ Concerning:

• fever/chills with pain
• worsening jaundice
• persistent itching disrupting sleep
• unintended weight loss

Symptom tracker

• itching (none/mild/moderate/severe)
• fever episodes (date/temp)
• belly pain (where, how severe)
• stool changes (if IBD present)
• energy level

5) 🏠 Home care and what helps (step-by-step)

✅ PSC requires specialist follow-up; home care focuses on symptom management and early recognition.

First 24–48 hours after diagnosis

✅ Do this now:

• Ask your specialist:
  • Does my child have large-duct PSC, small-duct PSC, or overlap?
  • Does my child need MRCP imaging?
  • What is the plan for IBD screening/monitoring?
  • How often are blood tests needed?
• Set up a fever plan:
  • “If fever + belly pain occurs, where do we go and who do we call?”

Supportive care at home

• hydration and rest during minor illness
• itch care:
  • moisturize
  • cool room at night
  • keep nails short
• healthy nutrition (especially if IBD coexists)

6) ⛔ What NOT to do (common mistakes)

• Don’t ignore fever with belly pain (could be cholangitis).
• Don’t stop medications without guidance.
• Don’t start supplements/herbs without approval.
• Don’t skip monitoring visits even if symptoms are mild.

7) 🚦 When to worry: triage guidance

🔴 Call 911 / Emergency now

• severe weakness, confusion, collapse
• vomiting blood or black stools
• severe belly pain with signs of shock

🟠 Same-day urgent visit

• fever + chills + right-upper belly pain
• jaundice worsening quickly
• persistent vomiting or dehydration

Example: “Fever with chills and new yellow eyes.”

🟡 Book a routine appointment

• new itching
• gradual fatigue
• questions about imaging or lab trends
• mild abdominal discomfort

🟢 Watch at home

• stable child with known PSC and no new symptoms, following plan

8) 🩺 How doctors diagnose it (what to expect)

What the clinician will ask

• symptoms (itching, fevers)
• bowel symptoms (IBD screening)
• medication history
• family autoimmune history

Physical exam basics

• liver and spleen size
• signs of jaundice
• growth and nutrition status

Possible tests (and why)

• blood tests:
  • liver enzymes (especially cholestatic pattern)
  • bilirubin
  • clotting and albumin
• MRCP (special MRI to map bile ducts)
• ultrasound
• autoimmune markers (supporting data)
• colonoscopy if IBD suspected or known PSC (team-directed)
• liver biopsy in selected cases

What tests are usually not needed

• repeated imaging without a clinical reason
• biopsy if MRCP and clinical picture are clear (depends on case)

9) 🧰 Treatment options

✅ There is no single “cure” medicine for PSC, but treatments focus on symptoms, complications, and overlapping IBD.

First-line management

• regular monitoring (labs + imaging)
• manage itching if present
• treat vitamin deficiencies if bile flow is reduced
• optimize IBD control if present

If not improving / complications develop

• treat cholangitis (bile duct infection) urgently with antibiotics
• endoscopic procedures if strictures/narrowings need treatment
• specialist hepatology care escalation

Severe cases (hospital care / transplant)

• progressive cirrhosis
• portal hypertension complications
• recurrent severe infections
• liver failure

Medication/treatment notes (parent-friendly)

• Itch medicines: reduce bile-related itching when needed
• Antibiotics: for cholangitis (urgent)
• Endoscopy procedures: may open narrowed ducts in some cases
• Transplant: for advanced disease or complications

10) ⏳ Expected course & prognosis

• PSC is typically chronic and needs long-term follow-up.
• Some children remain stable for many years.
• Others may have progression and need advanced care.

What “getting better” looks like

• stable labs
• fewer symptoms (itching, fatigue)
• fewer infections

What “getting worse” looks like

• increasing jaundice
• recurrent fevers/cholangitis
• worsening scarring signs on imaging
• complications of portal hypertension

11) ⚠️ Complications (brief but clear)

More common complications

• itching
• vitamin deficiencies (A, D, E, K)
• cholangitis (bile duct infection)

Serious complications

• cirrhosis
• portal hypertension (enlarged spleen, varices)
• variceal bleeding
• liver failure

12) 🛡️ Prevention and reducing future problems

• keep vaccines up to date (especially hepatitis)
• avoid alcohol later in adolescence/adulthood
• avoid smoking/vaping exposure
• early evaluation of fevers
• strong IBD control when present
• consistent follow-up

13) 🌟 Special situations

PSC with IBD

Common overlap:

• colon monitoring plans are important
• symptom tracking should include stool patterns and bleeding

Teens

Adherence and mental health support matter; chronic conditions can be stressful.

Kids with chronic conditions/immunosuppression

Lower threshold for evaluating fever.

Neurodevelopmental differences/autism

Use visual medication schedules; caregiver-supervised routines.

Travel considerations

Carry:

• medical summary
• medication list
• “fever + belly pain” emergency plan

School/daycare notes

Allow clinic visits; notify parent if fever or severe abdominal pain occurs.

14) 📅 Follow-up plan

• regular hepatology follow-up
• scheduled liver blood tests
• periodic imaging (MRCP/ultrasound as advised)
• IBD monitoring and colon surveillance if applicable
• urgent plan for fever/pain episodes

15) ❓ Parent FAQs

“Is it contagious?”

No.

“Can my child eat ___?”

Usually yes. If IBD is present, diet may be individualized.

“Can they bathe/swim/exercise?”

Yes, usually. Avoid contact sports if spleen is enlarged.

“Will they outgrow it?”

PSC is chronic; many children do well with monitoring and care.

“When can we stop treatment?”

Follow-up is long-term. Treatments may change over time; never stop medications without guidance.

16) 🧾 Printable tools (high-value add-ons)

🧾 Printable: PSC One-Page Action Plan

Daily/weekly:

• Take medications as prescribed
• Track itching and energy
• Monitor bowel symptoms if IBD present

Call clinic if:

• new itching
• worsening fatigue
• mild jaundice

Urgent/same-day care if:

• fever + chills
• fever + right-upper belly pain
• jaundice worsening quickly

ER if:

• vomiting blood
• black stools
• confusion/collapse

🧾 Printable: Fever & Pain Emergency Checklist

• Temperature: ______
• Pain location: ______
• Jaundice change: yes/no
• Vomiting: yes/no
• Who to call: ____________________
• Where to go: _____________________

🧾 Printable: “Red Flags” Fridge Sheet

⚠️ Urgent: fever + chills + belly pain, rapidly worsening jaundice, vomiting blood, black stools, confusion.

17) 📚 Credible sources + last updated date

Trusted references:

• Pediatric hepatology society educational resources
• Children’s hospital PSC family guides
• IBD and liver disease resources from major pediatric centers

Last reviewed/updated on: 2025-12-30
Local guidance may differ based on MRCP findings and IBD status.

🧡 Safety disclaimer

This guide supports—not replaces—medical care. If you are worried about your child, trust your instincts and seek urgent medical assessment.

This guide was fully developed & reviewed by Dr. Mohammad Hussein, MD, FRCPC ROYAL COLLEGE–CERTIFIED PEDIATRICIAN & PEDIATRIC GASTROENTEROLOGIST Board-certified pediatrician and pediatric gastroenterologist (Royal College of Physicians and Surgeons of Canada) with expertise in inflammatory bowel disease, eosinophilic gastrointestinal disorders, motility and functional testing, and complex nutrition across diverse international practice settings.

To book an online assessment Email Dr. Hussein’s Assistant Elizabeth Gray at: Elizabeth.Gray@pedsgimind.ca
In the email subject, please write: New Assessment Appointment with Dr. Hussein

Important: This appointment is completely online as Dr. Hussein is currently working overseas. This service is not covered by OHIP